Ewing's Sarcoma

Definition

Ewing's sarcoma is a
cancerous bone
tumor that may occur in any bone in the body. When it occurs in soft tissue, it is called extraosseous sarcoma. The most commonly affected areas include the pelvis, thigh, lower leg, upper arm, and chest wall.

Causes

The causes of Ewing's sarcoma are not fully understood. Chromosomal rearrangement that alters the genes may be a factor.

Risk Factors

Because the causes of the cancer are unknown, risk factors are also not fully understood. The following factors may increase your chance of developing Ewing's sarcoma:

Race: Caucasians

Age—Teenagers

Gender—Male

Symptoms

Symptoms include:

Pain, redness, and swelling surrounding the tumor

Fever

Weight loss and reduced appetite

Fatigue

Paralysis and incontinence in cases where the tumor is near the spinal cord

Numbness, tingling, and paralysis caused by compression of nerves by the tumor

Difficulty breathing if the tumor is in the chest wall

Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done. You will be treated by a team of doctors which may include an oncologist who specializes in cancer, an orthopaedic surgeon who operates on bones, and a radiation oncologist who works with radiation to kill cancer cells.

Treatment

Treatment involves a combination of chemotherapy, surgery, and radiation. Typically, patients are first treated with chemotherapy for 8-12 weeks, followed by a CT or MRI scan to evaluate the tumor. Depending on the tumor size and location, either surgery or radiation follows. Chemotherapy then resumes for several months after surgery or radiation.

Chemotherapy

Chemotherapy
drugs are used to kill tumor cells. The medications used to treat this tumor include
vincristine
(Oncovin)
,
dactinomycin
(Actinomycin D),
cyclophosphamide
(Cytoxan),
doxorubicin
(Adriamycin),
ifosfamide
(Ifex), and
etoposide
(VePesid). Your doctor will prescribe a combination of these drugs to be given over a set time. This usually means every 2-4 weeks for several months.

Surgery

Surgery may be used to remove the tumor, as well as rebuild the affected bone. Depending on the location of the tumor, a
bone graft
or prosthesis may be needed. There are special types of prostheses that expand as the bone grows. Sometimes, several surgeries are needed to make sure the limb functions properly.

Radiation Therapy

Radiation therapy
uses high-energy x-rays to kill tumor cells. It may be used with surgery or instead of surgery. It is usually given over several weeks and is followed by chemotherapy.

Future Therapy

A combination of high-dose chemotherapy and
stem cell transplant
is being evaluated at certain cancer centers. This combination therapy is typically used in cases where the tumor is very resistant.

Prevention

Ewing's sarcoma occurs for unknown reasons. There are currently no preventive measures to reduce the risk of developing the cancer.

Revision Information

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.